Lucy Perrem1,2,3,4,5, Sanja Stanojevic6,3, Michelle Shaw3, Renee Jensen1,3, Nancy McDonald1,3, Sarah M Isaac3, Miriam Davis7, Charles Clem7, Julia Guido3, Sylvia Jara7, Lisa France7, Melinda Solomon1,2,3, Hartmut Grasemann1,2,3, Valerie Waters8,2,3, Neil Sweezey1,2,3, Don B Sanders7, Stephanie D Davis9, Felix Ratjen1,2,3. 1. Division of Respiratory Medicine and. 2. Department of Paediatrics and. 3. Translational Medicine Program, SickKids Research Institute, Toronto, Ontario, Canada. 4. Royal College of Surgeons in Ireland, Dublin, Ireland. 5. National Children's Research Centre, Children's Health Ireland, Dublin, Ireland. 6. Institute of Health Policy Management and Evaluation, University of Toronto, Toronto, Ontario, Canada. 7. Division of Pediatric Pulmonology, Riley Hospital for Children, Indianapolis, Indiana; and. 8. Division of Infectious Diseases, The Hospital for Sick Children, Toronto, Ontario, Canada. 9. Division of Pediatric Pulmonology, Department of Pediatrics, University of North Carolina, Chapel Hill, North Carolina.
Abstract
Rationale: The lung clearance index (LCI) is responsive to acute respiratory events in preschool children with cystic fibrosis (CF), but its utility to identify and manage these events in school-age children with CF is not well defined. Objectives: To describe changes in LCI with acute respiratory events in school-age children with CF. Methods: In a multisite prospective observational study, the LCI and FEV1 were measured quarterly and during acute respiratory events. Linear regression was used to compare relative changes in LCI and FEV1% predicted at acute respiratory events. Logistic regression was used to compare the odds of a significant worsening in LCI and FEV1% predicted at acute respiratory events. Generalized estimating equation models were used to account for repeated events in the same subject.Measurements and Main Results: A total of 98 children with CF were followed for 2 years. There were 265 acute respiratory events. Relative to a stable baseline measure, LCI (+8.9%; 95% confidence interval, 6.5 to 11.3) and FEV1% predicted (-6.6%; 95% confidence interval, -8.3 to -5.0) worsened with acute respiratory events. A greater proportion of events had a worsening in LCI compared with a decline in FEV1% predicted (41.7% vs. 30.0%; P = 0.012); 53.9% of events were associated with worsening in LCI or FEV1. Neither LCI nor FEV1 recovered to baseline values at the next follow-up visit.Conclusions: In school-age children with CF, the LCI is a sensitive measure to assess lung function worsening with acute respiratory events and incomplete recovery at follow-up. In combination, the LCI and FEV1 capture a higher proportion of events with functional impairment.
Rationale: The lung clearance index (LCI) is responsive to acute respiratory events in preschool children with cystic fibrosis (CF), but its utility to identify and manage these events in school-age children with CF is not well defined. Objectives: To describe changes in LCI with acute respiratory events in school-age children with CF. Methods: In a multisite prospective observational study, the LCI and FEV1 were measured quarterly and during acute respiratory events. Linear regression was used to compare relative changes in LCI and FEV1% predicted at acute respiratory events. Logistic regression was used to compare the odds of a significant worsening in LCI and FEV1% predicted at acute respiratory events. Generalized estimating equation models were used to account for repeated events in the same subject.Measurements and Main Results: A total of 98 children with CF were followed for 2 years. There were 265 acute respiratory events. Relative to a stable baseline measure, LCI (+8.9%; 95% confidence interval, 6.5 to 11.3) and FEV1% predicted (-6.6%; 95% confidence interval, -8.3 to -5.0) worsened with acute respiratory events. A greater proportion of events had a worsening in LCI compared with a decline in FEV1% predicted (41.7% vs. 30.0%; P = 0.012); 53.9% of events were associated with worsening in LCI or FEV1. Neither LCI nor FEV1 recovered to baseline values at the next follow-up visit.Conclusions: In school-age children with CF, the LCI is a sensitive measure to assess lung function worsening with acute respiratory events and incomplete recovery at follow-up. In combination, the LCI and FEV1 capture a higher proportion of events with functional impairment.
Entities:
Keywords:
early CF lung disease; lung clearance index; lung function recovery; multiple breath washout; pulmonary exacerbations
Authors: Erick Forno; Steven H Abman; Jagdev Singh; Mary E Robbins; Hiran Selvadurai; Paul T Schumacker; Paul D Robinson Journal: Am J Respir Crit Care Med Date: 2021-08-01 Impact factor: 30.528